Aortopulmonary window. Experience of eleven cases.

Aortopulmonary (AP) window is a communication between the ascending aorta and the main pulmonary artery, in the presence of two separate arterial valves arising from separate subarterial ventricular outflow tracts. It is a rare anomaly that accounts for approximately 0.1% to 0.2% of all congenital heart disease. The purpose of this study was to assess clinical features, surgical treatment and outcome of patients with aortopulmonary window referred to a tertiary pediatric cardiac center over a 30-year period. Eleven patients were diagnosed with AP window, ten with the proximal type and one with the distal type defect. Age at first evaluation ranged from three days to 13 years (mean 44.5+/-63.3 months; median three months). Echocardiography enabled correct diagnosis in the four most recent cases. Seven patients underwent closure of the AP window through a transaortic approach and three patients underwent ligation. Simultaneously, significant associated cardiac anomalies were corrected in three patients: correction of interrupted aortic arch in two patients and closure of ventricular septal defect and Dacron patch enlargement of the right ventricular outflow tract in one patient. One patient was not operated because of fixed high pulmonary vascular resistance. Operative mortality was 10% (1/10) and there were no late deaths. Mean follow-up was 10+/-4.9 years. All nine surviving operated patients are asymptomatic, without medication, with no residual defects and without pulmonary hypertension. In conclusion, advances in diagnostic and surgical approaches to AP window in recent years have enabled earlier intervention with good outcome.